Pyrimidine analog of ATP. Activates chloride channels in epithelial cells; increases ciliary beat frequency and induces degranulation of goblet cells in airway epithelia. Also effects inflammatory cell function and vascular reactivity. Isoln from rabbit muscle: Lipton et al., J. Am. Chem. Soc. 75, 5450 (1953). Synthesis: Kenner et al., J. Chem. Soc. 1954, 2288; Hall, Khorana, J. Am. Chem. Soc. 76, 5056 (1954). Review of bioactivity mediated by nucleotide receptors: S. E. O'Connor, Life Sci. 50, 1657-1664 (1992). Clinical effect on chloride secretion in cystic fibrosis: M. R. Knowles et al., Am. J. Respir. Crit. Care Med. 151, S65 (1995); on mucociliary clearance in combination with amiloride, q.v.: W. D. Bennett et al., ibid. 153, 1796 (1996).
In treatment of cystic fibrosis.